Indeed, inhibition of autophagy paradoxically protects retinal ganglion cells (RGCs) from the consequences caused by expression of mutants of the key mitochondrial dynamics gene optic atrophy 1 (Opa1), mutated in the neurodegenerative disease autosomal dominant optic atrophy (ADOA), and corrects the visual defect caused by conditional RGC Opa1 ablation. The gene discussed is OPA1; the disease is autosomal dominant optic atrophy.